ILD
MCID: INT066
MIFTS: 59

Interstitial Lung Disease (ILD)

Categories: Blood diseases, Rare diseases, Respiratory diseases

Aliases & Classifications for Interstitial Lung Disease

MalaCards integrated aliases for Interstitial Lung Disease:

Name: Interstitial Lung Disease 12 74 52 58 15 17
Ild 12 52 58 15
Lung Diseases, Interstitial 43 71
Lung Diseases Interstitial 54
Interstitial Lung Diseases 42

Classifications:

Orphanet: 58  
Rare respiratory diseases


External Ids:

Disease Ontology 12 DOID:3082
MeSH 43 D017563
SNOMED-CT 67 64667001
ICD10 32 J84.9
MESH via Orphanet 44 D017563
UMLS via Orphanet 72 C0206062
Orphanet 58 ORPHA182095
UMLS 71 C0206062

Summaries for Interstitial Lung Disease

NIH Rare Diseases : 52 Interstitial lung diseases (ILDs) are a large group of disorders characterized by thickening of the walls between the alveoli , increased production and buildup of collagen , and pulmonary fibrosis (progressive scarring of lung tissue ). The scarring eventually affects the ability to breathe and get enough oxygen into the bloodstream. Symptoms typically include shortness of breath and a dry cough. There are numerous possible causes of ILD, including connective tissue disorders , autoimmune diseases (e.g. rheumatoid arthritis ), occupational exposures (such as asbestos), infection, and many drugs. Specific diseases leading to ILD may include sarcoidosis , pulmonary Langerhans cell histiocytosis , and eosinophilic pulmonary diseases . The list of substances and conditions that can lead to ILD is long. However, in many cases, the cause is not known. Some cases of unknown cause that share similar characteristics are called idiopathic interstitial pneumonias . Lung scarring that occurs in ILD is permanent. However, some treatments may temporarily improve symptoms or slow the disease's progress. Treatment options may include various medications, oxygen therapy , pulmonary rehabilitation , and as a last resort, lung transplantation .

MalaCards based summary : Interstitial Lung Disease, also known as ild, is related to interstitial pneumonitis, desquamative, familial and surfactant dysfunction, and has symptoms including shortness of breath, hemoptysis and snoring. An important gene associated with Interstitial Lung Disease is INPP5E (Inositol Polyphosphate-5-Phosphatase E), and among its related pathways/superpathways are ERK Signaling and PEDF Induced Signaling. The drugs Calcium carbonate and Oseltamivir have been mentioned in the context of this disorder. Affiliated tissues include lung, testes and t cells, and related phenotypes are Decreased viability with paclitaxel and Decreased viability with paclitaxel

Disease Ontology : 12 A lung disease that is characterized by inflammation and altered lung interstitium compromising pulmonary function and often has symptom shortness of breath, dyspnea, and/or cough.

MedlinePlus : 42 Interstitial lung disease is the name for a large group of diseases that inflame or scar the lungs. The inflammation and scarring make it hard to get enough oxygen. The scarring is called pulmonary fibrosis. Breathing in dust or other particles in the air is responsible for some types of interstitial lung diseases. Specific types include Black lung disease among coal miners, from inhaling coal dust Farmer's lung, from inhaling farm dust Asbestosis, from inhaling asbestos fibers Siderosis, from inhaling iron from mines or welding fumes Silicosis, from inhaling silica dust Other causes include autoimmune diseases or occupational exposures to molds, gases, or fumes. Some types of interstitial lung disease have no known cause. Treatment depends on the type of exposure and the stage of the disease. It may involve medicines, oxygen therapy, or a lung transplant in severe cases.

Wikipedia : 74 Interstitial lung disease (ILD), or diffuse parenchymal lung disease (DPLD), is a group of lung diseases... more...

Related Diseases for Interstitial Lung Disease

Diseases related to Interstitial Lung Disease via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 642)
# Related Disease Score Top Affiliating Genes
1 interstitial pneumonitis, desquamative, familial 34.2 SFTPD SFTPC SFTPB MUC1 ABCA3
2 surfactant dysfunction 33.6 SFTPC SFTPB ABCA3
3 surfactant metabolism dysfunction, pulmonary, 1 33.5 SFTPB ABCA3
4 pneumoconiosis 33.5 TGFB1 IL1A CXCL8 CCL2
5 pulmonary fibrosis 33.4 TGFB1 SFTPD SFTPC EDN1 CXCL8 CCL2
6 extrinsic allergic alveolitis 33.4 SFTPD CXCL8 CCL5 CCL2
7 idiopathic interstitial pneumonia 33.4 TGFB1 SFTPD SFTPC SFTPB MUC1 EDN1
8 sarcoidosis 1 32.4 SFTPD IL1A CCL5 CCL2
9 connective tissue disease 32.3 SFTPD MUC1 IL1A EDN1 CXCL8 CCL2
10 lung disease 32.2 TGFB1 SFTPD SFTPC SFTPB NKX2-1 NARS1
11 bronchiolitis 32.2 SFTPD CXCL8 CCL5 CCL2
12 myositis 32.1 NARS1 IL1A IFIH1 CXCL8
13 nonspecific interstitial pneumonia 32.1 TGFB1 SFTPD SFTPC MUC1 IFIH1 CCL2
14 cryptogenic organizing pneumonia 32.0 SFTPD SFTPC MUC1 CXCL8
15 pulmonary fibrosis, idiopathic 31.9 TGFB1 SFTPD SFTPC SFTPB MUC1 IL1A
16 bronchiolitis obliterans 31.8 SFTPD EDN1 CXCL8
17 allergic hypersensitivity disease 31.7 SFTPD CXCL8 CCL5 CCL17
18 exanthem 31.7 IFIH1 EGFR CXCL8
19 acute interstitial pneumonia 31.7 SFTPD SFTPC SFTPB MUC1 IFIH1
20 pulmonary disease, chronic obstructive 31.6 TGFB1 SFTPD SFTPB CXCL8 CCL5 CCL2
21 eosinophilic pneumonia 31.6 SFTPD MUC1 CXCL8 CCL5 CCL17
22 pulmonary sarcoidosis 31.5 SFTPD CCL5 CCL2
23 lung cancer susceptibility 3 31.4 TGFB1 SFTPD SFTPC SFTPB NKX2-1 MUC1
24 pleuroparenchymal fibroelastosis 31.4 SFTPD MUC1
25 silicosis 31.4 TGFB1 SFTPD IL1A CXCL8 CCL2
26 pulmonary alveolar proteinosis 31.4 SFTPD SFTPC SFTPB MUC1 CXCL8 CCL2
27 arthritis 31.4 TGFB1 IL1A CXCL8 CCL5 CCL2
28 pulmonary emphysema 31.2 SFTPD SFTPC CXCL8
29 adult respiratory distress syndrome 31.2 SFTPD SFTPC SFTPB CXCL8
30 pneumonia 31.2 TGFB1 SFTPD SFTPC SFTPB MUC1 CXCL8
31 sleep apnea 31.1 EDN1 CXCL8 CCL2
32 autoimmune disease 31.1 TNFRSF13B TGFB1 IL1A IFIH1 CXCL8 CCL5
33 bacterial pneumonia 31.1 SFTPD SFTPB CXCL8
34 proteasome-associated autoinflammatory syndrome 1 31.0 IL1A CXCL8 CCL5 CCL2
35 pleurisy 31.0 TGFB1 CXCL8 CCL2
36 aspergillosis 30.9 SFTPD CXCL8 CCL5 CCL17
37 psoriasis 30.9 IL1A IFIH1 CXCL8 CCL5 CCL2 CCL17
38 small cell carcinoma 30.9 NKX2-1 MUC1 EGFR
39 viral pneumonia 30.8 SFTPD CXCL8 CCL5 CCL2
40 diffuse pulmonary fibrosis 30.8 SFTPC ABCA3
41 pulmonary eosinophilia 30.7 SFTPD CXCL8 CCL5 CCL17
42 interstitial emphysema 30.7 SFTPC SFTPB ABCA3
43 lipid pneumonia 30.7 SFTPB MUC1
44 cystic fibrosis 30.7 TGFB1 SFTPD SFTPC CXCL8 CCL17
45 stachybotrys chartarum 30.6 IL1A CCL5 CCL2
46 sclerosing hemangioma 30.6 SFTPB NKX2-1 MUC1
47 bird fancier's lung 30.6 SFTPD MUC1
48 cytomegalovirus infection 30.6 CXCL8 CCL5 CCL2
49 keratoconjunctivitis 30.6 CXCL8 CCL5 CCL2
50 skin disease 30.6 SFTPD ITGA3 IL1A CXCL8 CCL17

Graphical network of the top 20 diseases related to Interstitial Lung Disease:



Diseases related to Interstitial Lung Disease

Symptoms & Phenotypes for Interstitial Lung Disease

Symptoms:

12
  • shortness of breath

UMLS symptoms related to Interstitial Lung Disease:


hemoptysis, snoring, coughing

GenomeRNAi Phenotypes related to Interstitial Lung Disease according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased viability with paclitaxel GR00179-A-1 8.92 EGFR TGFB1
2 Decreased viability with paclitaxel GR00179-A-3 8.92 EGFR TGFB1

MGI Mouse Phenotypes related to Interstitial Lung Disease:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 immune system MP:0005387 10 CCL17 CCL2 CCL5 EGFR IFIH1 IL1A
2 neoplasm MP:0002006 9.7 EGFR IL1A ITGA3 NKX2-1 SFTPC TGFB1
3 renal/urinary system MP:0005367 9.56 ABCA3 EDN1 EGFR IFIH1 INPP5E ITGA3
4 respiratory system MP:0005388 9.23 ABCA3 EGFR ITGA3 NKX2-1 SFTPB SFTPC

Drugs & Therapeutics for Interstitial Lung Disease

Drugs for Interstitial Lung Disease (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 296)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Calcium carbonate Approved, Investigational Phase 4 471-34-1
2
Oseltamivir Approved Phase 4 196618-13-0, 204255-11-8 65028
3
Ledipasvir Approved Phase 4 1256388-51-8 67505836
4
Sofosbuvir Approved Phase 4 1190307-88-0 45375808
5
Propofol Approved, Investigational, Vet_approved Phase 4 2078-54-8 4943
6
Iloprost Approved, Investigational Phase 4 78919-13-8 6443959
7
Propranolol Approved, Investigational Phase 4 525-66-6 4946
8
Nortriptyline Approved Phase 4 72-69-5 4543
9
Ergotamine Approved Phase 4 113-15-5 8223
10
Sumatriptan Approved, Investigational Phase 4 103628-46-2 5358
11
Entecavir Approved, Investigational Phase 4 142217-69-4 153941
12
Lamivudine Approved, Investigational Phase 4 134678-17-4 60825
13
Peginterferon alfa-2b Approved Phase 4 215647-85-1, 99210-65-8
14
Sevoflurane Approved, Vet_approved Phase 4 28523-86-6 5206
15
Calcium Approved, Nutraceutical Phase 4 7440-70-2 271
16 Gallopamil Investigational Phase 4 16662-47-8
17
Lactitol Investigational Phase 4 585-86-4, 585-88-6 493591
18
Adefovir Investigational Phase 4 106941-25-7
19
Tenofovir Experimental, Investigational Phase 4 147127-20-6 464205
20 Anti-Ulcer Agents Phase 4
21 Calcium, Dietary Phase 4
22 Antacids Phase 4
23 interferons Phase 4
24 Anti-Infective Agents Phase 4
25 Immunologic Factors Phase 4
26 Antiviral Agents Phase 4
27 Calcineurin Inhibitors Phase 4
28 Ledipasvir, sofosbuvir drug combination Phase 4
29 Anesthetics, Intravenous Phase 4
30 Interferon-alpha Phase 4
31 Interferon alpha-2 Phase 4
32 Platelet Aggregation Inhibitors Phase 4
33
Minocycline Approved, Investigational Phase 3 10118-90-8 5281021
34
Mycophenolic acid Approved Phase 3 24280-93-1 446541
35
Sirolimus Approved, Investigational Phase 3 53123-88-9 5284616 6436030 46835353
36
Miconazole Approved, Investigational, Vet_approved Phase 3 22916-47-8 4189
37
Everolimus Approved Phase 3 159351-69-6 6442177 70789204
38
Basiliximab Approved, Investigational Phase 3 179045-86-4, 152923-56-3
39
Lactulose Approved Phase 3 4618-18-2 11333
40
Tadalafil Approved, Investigational Phase 3 171596-29-5 110635
41
Osimertinib Approved Phase 3 1421373-65-0 71496458
42
Macitentan Approved Phase 2, Phase 3 441798-33-0
43
Bismuth subsalicylate Approved, Vet_approved Phase 3 14882-18-9 53629521
44
Nintedanib Approved Phase 3 656247-17-5 56843413
45
Treprostinil Approved, Investigational Phase 2, Phase 3 81846-19-7 54786 6918140
46
Selexipag Approved Phase 2, Phase 3 475086-01-2
47
Denosumab Approved Phase 3 615258-40-7
48
Risedronate Approved, Investigational Phase 3 105462-24-6 5245
49
Zoledronic Acid Approved Phase 3 118072-93-8 68740
50
Acetylcysteine Approved, Investigational Phase 2, Phase 3 616-91-1 12035

Interventional clinical trials:

(show top 50) (show all 407)
# Name Status NCT ID Phase Drugs
1 Randomized Controlled Trial of Pirfenidone in Patients With Progressive Interstitial Lung Disease Associated With Clinically Amyopathic Dermatomyositis Unknown status NCT02821689 Phase 4 Pirfenidone
2 Patients With Pulmonary Hypertension or Interstitial Lung Disease Travelling to Altitude - Effect of Nocturnal Oxygen Therapy on Breathing and Sleep Unknown status NCT02150616 Phase 4 Oxygen;Sham oxygen (room air)
3 Use of the Endothelin-1 Antagonist Bosentan in Patients With Established Pulmonary Hypertension and Fibrotic Lung Disease. - A Randomised, Placebo-Controlled, Double-Blinded Study. Unknown status NCT00637065 Phase 4 Bosentan;Placebo
4 Cyclosporine A in the TReatment of Interstitial Pneumonitis Associated With Sjogren's Syndrome(CTRIPS): A Prospective, Randomized, Multicenter, Double-Blind Placebo-Controlled Trial Unknown status NCT02370550 Phase 4 Cyclosporin A;Prednisone;Placebo;Calcium carbonate D
5 Interstitial Fibrosis in Protocol Biopsies of Renal Allografts: A Prospective, Randomised Trial of Sirolimus Versus Cyclosporine.(Fibrasic) Unknown status NCT00493194 Phase 4 sirolimus;cyclosporine;daclizumab
6 Banlangen Granules Anti-seasonal Influenza Study: a Randomized, Double Blind, Positive and Placebo Controlled,Clinical Study. Unknown status NCT02232945 Phase 4 placebo of oseltamivir phosphate;oseltamivir phosphate;Banlangen (Radix Isatidis) granules;placebo of Banlangen(Radix Isatidis) granules
7 Effects of Pulmonary Rehabilitation in Interstitial Lung Diseases: a Prospective Randomized Controlled Trial Completed NCT00882817 Phase 4
8 Palliation of Dyspnea With Morphine in Patients With Interstitial Lung Disease Completed NCT02622022 Phase 4 Morphine hydrochloride
9 A Multicenter, Open-label Study of Harvoni ® (Sofosbuvir Ledipasvir Fixed Dose Combination) in Subjects Infected With Chronic Hepatitis C and Advanced Heart Failure or Lung Disease Completed NCT02858180 Phase 4 Sofosbuvir/ledipasvir fixed dose combination(SOF/LDV FDC)
10 Safety and Efficacy of Everolimus Transition in Minimizing Progressive Graft Dysfunction and Interstitial Fibrosis in Adult Kidney Transplant Recipients Completed NCT02096107 Phase 4 Everolimus
11 Target-controlled Versus Manually-controlled Propofol Sedation in Flexible Bronchoscopy. A Randomized Non-inferiority Trial. Completed NCT02246023 Phase 4
12 Inhaled Iloprost for Sarcoidosis Associated Pulmonary Hypertension Completed NCT00403650 Phase 4 Iloprost
13 Cupping Therapy and SERKANGABIN Versus Conventional Treatment of Migraine Headache: a Randomized Controlled Trial Completed NCT01476930 Phase 4 conventional migraine drug treatment
14 SWITCH OR ADD PEGYLATED-INTERFERON IN CHRONIC HEPATITIS B PATIENTS ON LONG TERM NUCLEOS(T)IDE THERAPY (SWAP TRIAL) Completed NCT01928511 Phase 4 peg-interferon alpha 2b, 1.5mcg/kg s/c given weekly;Nucleos(t)ide analogue therapy
15 Efficacy and Safety of Two Glucocorticoid Regimens in the Treatment of Sarcoidosis: a Randomized Controlled Trial Recruiting NCT03265405 Phase 4 Low dose prednisolone;Medium dose prednisolone
16 REgistry-based Randomized Controlled Trial of Treatment Duration and Mortality in Long-term OXygen Therapy (REDOX) A Multicenter, Phase IV, Registry-Based, Randomized Controlled Trial (R-RCT) Recruiting NCT03441204 Phase 4 LTOT 24 h/day;LTOT 15h/day
17 Patients With Pulmonary Hypertension or Interstitial Lung Disease Travelling to Altitude - Effect of Nocturnal Oxygen Therapy on Exercise Performance Active, not recruiting NCT02143687 Phase 4 Oxygen;Sham oxygen (room air)
18 Randomized Open-label Study of the Impact of Prolonged Systemic Corticosteroid Therapy on the Course and Relapse Risk of Checkpoint Inhibitor Interstitial Lung Disease (Pneumonitis) Related to the Treatment of Solid Tumors With Anti-programmed-death Type 1 Receptor or Ligand Antibodies Not yet recruiting NCT04036721 Phase 4 Prolonged glucocorticosteroid (prednisone) regimen;Short glucocorticosteroid (prednisone) regimen
19 Randomized Placebo-Controlled Study of Sildenafil For The Treatment of Pulmonary Hypertension Secondary to Idiopathic Pulmonary Fibrosis: A Pilot Study Withdrawn NCT00625079 Phase 4 sildenafil
20 Treatment of Pulmonary Arterial Hypertension Secondary to Idiopathic Pulmonary Hypertension With Bosentan: A Single Center Pilot Study Withdrawn NCT00625469 Phase 4 bosentan
21 The NoTube Study: Evaluation of the Necessity of a Chest Tube After a Video-assisted Thoracoscopic Surgery Pulmonary Wedge Resection. Unknown status NCT00841750 Phase 3
22 Minocycline Treatment in Patients With Idiopathic Pulmonary Fibrosis Being Treated With Standard of Care Therapy- a Pilot Study Unknown status NCT00203697 Phase 3 minocycline
23 Long-term Open-label Study in Patients With Interstitial Lung Disease Associated With Systemic Sclerosis Who Completed the Protocol AC-052-330. Completed NCT00319033 Phase 2, Phase 3 bosentan
24 A Double Blind, Randomized, Placebo-controlled Trial Evaluating the Efficacy and Safety of Nintedanib Over 52 Weeks in Patients With Progressive Fibrosing Interstitial Lung Disease (PF-ILD) Completed NCT02999178 Phase 3 Nintedanib;Placebo
25 Rituximab for the Treatment of Rheumatoid Arthritis-Associated Interstitial Pneumonia: A Pilot Study Completed NCT00578565 Phase 3 Rituximab
26 Intravenous Cyclophosphamide for the Treatment of Systemic Sclerosis Associated Interstitial Lung Disease Completed NCT01570764 Phase 3 Cyclophosphamide;Placebo
27 A Randomized Controlled Trial to Compare the Efficacy of Oral Mycophenolate Mofetil With Placebo in Patients With Systemic Sclerosis Related Early Interstitial Lung Disease Completed NCT02896205 Phase 3 Mycophenolate mofetil;Placebo
28 Rituximab in Life Threatening Therapy Resistant Progressive Interstitial Pneumonitis Completed NCT02251964 Phase 2, Phase 3 Rituximab
29 "A RCT ON CLINICAL EFFICACY OF 1% vs. 2% LIGNOCAINE IN COUGH SUPPRESSION AND PAIN RELIEF IN PATIENTS UNDERGOING FLEXIBLE BRONCHOSCOPY" Completed NCT01955824 Phase 2, Phase 3 1% lignocaine;2% lignocaine
30 A Double Blind Randomized Control Trial of Tadalafil in Interstitial Lung Disease of Scleroderma Completed NCT01553981 Phase 3 Tadalafil;Placebo
31 An Open-label Clinical Trial of the Combination Treatment of Tacrolimus and Corticosteroid in Polymyositis/Dermatomyositis Patients With Interstitial Pneumonitis, With Comparison Against Corticosteroid-treated Historical Controls Completed NCT00504348 Phase 2, Phase 3 Tacrolimus
32 Prospective, Multicenter, Randomized Open Study to Evaluate the Progression of Renal Graft Fibrosis According to the Epithelial-mesenchymal Transition (EMT) in de Novo Renal Transplant Recipients Treated Either by a CNI Free Immunosuppressive Regimen With Everolimus and Enteric-coated Mycophenolate Sodium or a CNI Based Regimen With Cyclosporine and Enteric-coated Mycophenolate Sodium Completed NCT01079143 Phase 3 Certican®;Neoral;Myfortic;Simulect®;Corticosteroids
33 A Double-blind, Randomized, Placebo-controlled, Multicenter Study to Assess the Efficacy, Safety and Tolerability of Bosentan in Patients With Interstitial Lung Disease Associated With Systemic Sclerosis Completed NCT00070590 Phase 2, Phase 3 Bosentan
34 A Double Blind, Randomised, Placebo-controlled Trial Evaluating Efficacy and Safety of Oral Nintedanib Treatment for at Least 52 Weeks in Patients With Systemic Sclerosis Associated Interstitial Lung Disease (SSc-ILD) Completed NCT02597933 Phase 3 Nintedanib;Placebo
35 Effects of Bosentan on Morbidity and Mortality in Patients With Idiopathic Pulmonary Fibrosis - a Multicenter, Double-blind, Randomized, Placebo-controlled, Parallel Group, Event-driven, Group Sequential, Phase III Study. Completed NCT00391443 Phase 3 Bosentan;Placebo
36 Open-Label Extension Study in Patients With Idiopathic Pulmonary Fibrosis Who Completed Protocol AC-052-321 (NCT00391443) Completed NCT00631475 Phase 3 Bosentan
37 A Double-blind, Randomized, Placebo-controlled, Multicenter Study to Assess the Efficacy, Safety, and Tolerability of Bosentan in Patients With Idiopathic Pulmonary Fibrosis, Open Label Extension Completed NCT00071461 Phase 2, Phase 3 bosentan;Placebo
38 ARIES-3: A Phase 3, Long-Term, Open-Label, Multicenter Safety and Efficacy Study of Ambrisentan in Subjects With Pulmonary Hypertension Completed NCT00380068 Phase 3 Ambrisentan
39 A Double-Blind, Placebo-Controlled, Randomized Study of the Efficacy (Gleevec Imatinib Mesylate) in Patients With Idiopathic Pulmonary Fibrosis Completed NCT00131274 Phase 2, Phase 3 Imatinib Mesylate (Gleevec)
40 Cyclophosphamide Versus Placebo in Scleroderma Lung Study Completed NCT00004563 Phase 3 Cyclophosphamide;Placebo
41 Endosonography (EUS and EBUS) vs Conventional Bronchoscopy for the Diagnosis of Sarcoidosis: a Randomized Trial Completed NCT00872612 Phase 3
42 Open Label, Multinational, Multicenter, Real World Treatment Study of Single Agent AZD9291 for Patients With Advanced/Metastatic Epidermal Growth Factor Receptor (EGFR) T790M Mutation-Positive Non-Small Cell Lung Cancer (NSCLC) Who Have Received Prior Therapy With an EGFR Tyrosine Kinase Inhibitor (EGFR-TKI) Completed NCT02474355 Phase 3 AZD9291 Dosing
43 Effects of Roflumilast in Hospitalized COPD on Mortality and Re-hospitalization Completed NCT01973998 Phase 3 Roflumilast;Placebo
44 An Open-label Extension Trial of the Long Term Safety of Nintedanib in Patients With 'Systemic Sclerosis Associated Interstitial Lung Disease' (SSc-ILD) Recruiting NCT03313180 Phase 3 Nintedanib
45 A Phase III, Randomized, Double-blind, Placebo Controlled, Multicenter Clinical Trial to Evaluate the Efficacy and Safety of Pirfenidone in Subjects With Dermatomyositis Interstitial Lung Disease (Dm-ILD) Recruiting NCT03857854 Phase 3 Pirfenidone;Placebos
46 A Double Blind, Randomised, Placebo-controlled Trial to Evaluate the Dose-exposure and Safety of Nintedanib Per os on Top of Standard of Care for 24 Weeks, Followed by Open Label Treatment With Nintedanib of Variable Duration, in Children and Adolescents (6 to 17 Year-old) With Clinically Significant Fibrosing Interstitial Lung Disease. Recruiting NCT04093024 Phase 3 Nintedanib (Ofev®);Placebo
47 A Phase III, Randomized, Double-blind, Placebo Controlled, Multicenter Clinical Trial to Evaluate the Efficacy and Safety of Pirfenidone in Subjects With Systemic Sclerosis-associated Interstitial Lung Disease (SSc-ILD) Recruiting NCT03856853 Phase 3 Pirfenidone
48 A Randomized, Double Blind Controlled Trial Comparing Rituximab Against Intravenous Cyclophosphamide in Connective Tissue Disease Associated Interstitial Lung Disease Recruiting NCT01862926 Phase 2, Phase 3 Rituximab;Cyclophosphamide
49 A Multicenter, Randomized, Double-Blinded, Placebo-Controlled Trial to Evaluate the Safety and Efficacy of Inhaled Treprostinil in Subjects With Pulmonary Hypertension Due to Parenchymal Lung Disease Recruiting NCT02630316 Phase 2, Phase 3 Inhaled Treprostinil;Placebo
50 CompRehensive Phenotypic Characterization of Patients With Scleroderma-Associated Interstitial Lung DiseasE and Pulmonary Hypertension (PH): The CRuSADE PH Study Recruiting NCT03726398 Phase 2, Phase 3 Opsumit 10 Mg Tablet

Search NIH Clinical Center for Interstitial Lung Disease

Cochrane evidence based reviews: lung diseases, interstitial

Genetic Tests for Interstitial Lung Disease

Anatomical Context for Interstitial Lung Disease

MalaCards organs/tissues related to Interstitial Lung Disease:

40
Lung, Testes, T Cells, Bone, B Cells, Heart, Skin

Publications for Interstitial Lung Disease

Articles related to Interstitial Lung Disease:

(show top 50) (show all 8192)
# Title Authors PMID Year
1
Imaging of Diffuse Lung Disease in the Intensive Care Unit Patient. 42
31731896 2020
2
Rituximab in treatment of anti-GBM antibody glomerulonephritis: A case report and literature review. 42
31689860 2019
3
New advances in the management of pulmonary sarcoidosis. 42
31641045 2019
4
Characteristics of disorders associated with genetic mutations of surfactant protein C. 54 61
20403820 2010
5
Alveolar surfactant homeostasis and the pathogenesis of pulmonary disease. 54 61
19824815 2010
6
[Interstitial lung disease associated with surfactant protein B and C deficiencies]. 54 61
20461691 2010
7
[Interstitial lung disease in the course of the surfactant protein C deficiency coexisting with the primary humoral immunodeficiency - case study]. 54 61
20461694 2010
8
[Relationship between the level of MCP-1 expression in sera of RA patients and ILD]. 54 61
20056091 2010
9
N-acetylcysteine inhibits IL-8 and MMP-9 release and ICAM-1 expression by bronchoalveolar cells from interstitial lung disease patients. 54 61
20360623 2010
10
Meckel-Gruber syndrome protein MKS3 is required for endoplasmic reticulum-associated degradation of surfactant protein C. 54 61
19815549 2009
11
New surfactant protein C gene mutations associated with diffuse lung disease. 54 61
19443464 2009
12
Genetic disorders of surfactant dysfunction. 54 61
19220077 2009
13
Surfactant protein C-deficient mice are susceptible to respiratory syncytial virus infection. 54 61
19304906 2009
14
Surfactant protein D and KL-6 as serum biomarkers of interstitial lung disease in patients with scleroderma. 54 61
19286849 2009
15
Bronchoalveoloar lavage fluid cytokines and chemokines as markers and predictors for the outcome of interstitial lung disease in systemic sclerosis patients. 54 61
19615053 2009
16
[EGFR and gefitinib (Iressa)]. 54 61
18633244 2008
17
ERdj4 and ERdj5 are required for endoplasmic reticulum-associated protein degradation of misfolded surfactant protein C. 54 61
18400946 2008
18
[The serum levels of cytokines in patients with rheumatoid arthritis associated interstitial lung disease and their clinical significance]. 54 61
18846962 2008
19
Genetic polymorphisms in the surfactant proteins in systemic sclerosis in Japanese: T/T genotype at 1580 C/T (Thr131Ile) in the SP-B gene reduces the risk of interstitial lung disease. 54 61
18263595 2008
20
Aberrant processing forms of lung surfactant proteins SP-B and SP-C revealed by high-resolution mass spectrometry. 54 61
19136726 2008
21
Misfolded BRICHOS SP-C mutant proteins induce apoptosis via caspase-4- and cytochrome c-related mechanisms. 54 61
17586700 2007
22
Independent review of interstitial lung disease associated with death in TRIBUTE (paclitaxel and carboplatin with or without concurrent erlotinib) in advanced non-small cell lung cancer. 54 61
17545850 2007
23
Genetic disorders of surfactant proteins. 54 61
17575475 2007
24
Inherited surfactant protein-B deficiency and surfactant protein-C associated disease: clinical features and evaluation. 54 61
17142157 2006
25
Thalidomide reduces IL-18, IL-8 and TNF-alpha release from alveolar macrophages in interstitial lung disease. 54 61
16837501 2006
26
The Brichos domain-containing C-terminal part of pro-surfactant protein C binds to an unfolded poly-val transmembrane segment. 54 61
16709565 2006
27
Side effects of anti-cancer molecular-targeted therapies (not monoclonal antibodies). 54 61
16721122 2006
28
Genetics of pediatric interstitial lung disease. 54 61
16721150 2006
29
Relationship between epidermal growth factor receptor gene mutations and the severity of adverse events by gefitinib in patients with advanced non-small cell lung cancer. 54 61
16503086 2006
30
Interstitial lung disease associated with gefitinib. 54 61
16137874 2006
31
Interstitial lung disease in patients with non-small-cell lung cancer treated with epidermal growth factor receptor inhibitors. 54 61
16720916 2006
32
Pulmonary surfactant proteins A and D: innate immune functions and biomarkers for lung diseases. 54 61
16472150 2006
33
Adaptation and increased susceptibility to infection associated with constitutive expression of misfolded SP-C. 54 61
16449190 2006
34
Familial interstitial lung disease in two young Korean sisters. 54 61
16361824 2005
35
Involvement of eicosanoids and surfactant protein D in extrinsic allergic alveolitis. 54 61
16319337 2005
36
[Genetic basis in chronic interstitial familial pneumopathy. Familial study of SFTPC]. 54 61
16910460 2005
37
[Neonatal respiratory failure associated with mutation in the surfactant protein C gene]. 54 61
15737281 2005
38
Nonspecific interstitial pneumonia, alveolar proteinosis, and abnormal proprotein trafficking resulting from a spontaneous mutation in the surfactant protein C gene. 54 61
15557112 2005
39
Serum KL-6 and surfactant proteins A and D in pediatric interstitial lung disease. 54 61
15654008 2005
40
Interstitial lung disease in a baby with a de novo mutation in the SFTPC gene. 54 61
15293602 2004
41
Matrix proteoglycans and remodelling of interstitial lung tissue in lymphangioleiomyomatosis. 54 61
15141380 2004
42
Progressive lung disease and surfactant dysfunction with a deletion in surfactant protein C gene. 54 61
14656744 2004
43
Augmented pulmonary IL-4 and IL-13 receptor subunit expression in idiopathic interstitial pneumonia. 54 61
15113854 2004
44
Mutation of SFTPC in infantile pulmonary alveolar proteinosis with or without fibrosing lung disease. 54 61
15039969 2004
45
Expression of a human surfactant protein C mutation associated with interstitial lung disease disrupts lung development in transgenic mice. 54 61
14525980 2003
46
[Respiratory diseases associated to surfactant proteins B and C deficiency]. 54 61
15279365 2003
47
Endothelin-1 levels in interstitial lung disease patients during sleep. 54 61
14569522 2003
48
Serum levels of surfactant protein D are increased in mice with lung tumors. 54 61
14522914 2003
49
Increased spontaneous interleukin-10 release from alveolar macrophages in active pulmonary sarcoidosis. 54 61
12746045 2003
50
Deletion of exon 4 from human surfactant protein C results in aggresome formation and generation of a dominant negative. 54 61
12538769 2003

Variations for Interstitial Lung Disease

ClinVar genetic disease variations for Interstitial Lung Disease:

6 ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎
# Gene Name Type Significance ClinVarId dbSNP ID GRCh37 Pos GRCh38 Pos
1 INPP5E NM_019892.6(INPP5E):c.1132C>T (p.Arg378Cys)SNV Pathogenic/Likely pathogenic 400 rs121918130 9:139327634-139327634 9:136433182-136433182
2 TNFRSF13B NM_012452.2(TNFRSF13B):c.310T>C (p.Cys104Arg)SNV risk factor 5302 rs34557412 17:16852187-16852187 17:16948873-16948873
3 SFTPC NM_003018.4(SFTPC):c.163C>T (p.Leu55Phe)SNV Likely pathogenic 598978 rs1563221666 8:22020207-22020207 8:22162694-22162694
4 ERF NM_006494.4(ERF):c.1636C>T (p.Arg546Ter)SNV Uncertain significance 599007 rs764412749 19:42752628-42752628 19:42248476-42248476
5 VANGL1 NM_138959.3(VANGL1):c.523C>T (p.Arg175Trp)SNV Uncertain significance 167818 rs142594314 1:116206600-116206600 1:115663979-115663979
6 DES NM_001927.4(DES):c.407T>A (p.Leu136His)SNV Uncertain significance 44261 rs397516695 2:220283591-220283591 2:219418869-219418869
7 GATA4 NM_002052.5(GATA4):c.94G>C (p.Ala32Pro)SNV Uncertain significance 599008 rs773545065 8:11565915-11565915 8:11708406-11708406

Expression for Interstitial Lung Disease

Search GEO for disease gene expression data for Interstitial Lung Disease.

Pathways for Interstitial Lung Disease

Pathways related to Interstitial Lung Disease according to GeneCards Suite gene sharing:

(show all 25)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.73 TNFRSF13B TGFB1 MUC1 ITGA3 IL1A EGFR
2
Show member pathways
13.59 TNFRSF13B TGFB1 IL1A EGFR EDN1 CXCL8
3
Show member pathways
13.44 TGFB1 ITGA3 IL1A EGFR CXCL8 CCL5
4
Show member pathways
13.28 TNFRSF13B TGFB1 ITGA3 IL1A EGFR CXCL8
5
Show member pathways
13.26 TNFRSF13B TGFB1 MUC1 IL1A EGFR CXCL8
6
Show member pathways
13.18 TNFRSF13B TGFB1 IL1A EGFR CXCL8 CCL5
7
Show member pathways
13.01 TGFB1 SFTPD IFIH1 EGFR CXCL8 CCL5
8 12.74 TGFB1 ITGA3 EGFR EDN1 CXCL8
9
Show member pathways
12.72 IL1A IFIH1 CXCL8 CCL5 CCL2
10
Show member pathways
12.23 CXCL8 CCL5 CCL2 CCL17
11
Show member pathways
12.13 IL1A EGFR EDN1 CXCL8
12 11.85 TGFB1 IL1A EGFR CXCL8 CCL2
13 11.78 TGFB1 MUC1 IL1A CXCL8 CCL2
14
Show member pathways
11.77 SFTPD SFTPC SFTPB
15 11.65 TGFB1 IL1A EDN1 CXCL8 CCL2
16 11.61 TGFB1 CCL5 CCL2 CCL17
17 11.52 TGFB1 IL1A CXCL8 CCL5 CCL2
18 11.45 TGFB1 CXCL8 CCL2
19 11.42 IL1A CXCL8 CCL5 CCL2
20
Show member pathways
11.34 SFTPD SFTPC SFTPB ABCA3
21 11.33 MUC1 ITGA3 CCL5
22 11.22 TNFRSF13B TGFB1 CXCL8
23
Show member pathways
11.07 SFTPD SFTPC SFTPB
24 10.9 TGFB1 SFTPC EDN1 CXCL8 CCL5 CCL2
25 10.46 TGFB1 ITGA3 IL1A EGFR CXCL8 CCL5

GO Terms for Interstitial Lung Disease

Cellular components related to Interstitial Lung Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.73 TGFB1 SFTPD SFTPC SFTPB MUC1 IL1A
2 extracellular space GO:0005615 9.44 TGFB1 SFTPD SFTPC SFTPB MUC1 IL1A
3 lamellar body GO:0042599 9.37 SFTPC SFTPB
4 clathrin-coated endocytic vesicle GO:0045334 9.33 SFTPD SFTPC SFTPB
5 multivesicular body lumen GO:0097486 9.32 SFTPC SFTPB
6 alveolar lamellar body GO:0097208 9.26 SFTPB ABCA3

Biological processes related to Interstitial Lung Disease according to GeneCards Suite gene sharing:

(show all 35)
# Name GO ID Score Top Affiliating Genes
1 immune response GO:0006955 10.04 IL1A CXCL8 CCL5 CCL2 CCL17
2 MAPK cascade GO:0000165 9.95 TGFB1 EGFR CCL5 CCL2
3 positive regulation of cell migration GO:0030335 9.95 TGFB1 EGFR EDN1 CCL5
4 cell surface receptor signaling pathway GO:0007166 9.93 TNFRSF13B EGFR EDN1 CCL2
5 cellular protein metabolic process GO:0044267 9.92 SFTPD SFTPC SFTPB ABCA3
6 inflammatory response GO:0006954 9.91 TGFB1 IL1A CXCL8 CCL5 CCL2 CCL17
7 chemotaxis GO:0006935 9.88 CXCL8 CCL5 CCL2 CCL17
8 positive regulation of ERK1 and ERK2 cascade GO:0070374 9.85 TGFB1 EGFR CCL5 CCL2 CCL17
9 lung development GO:0030324 9.84 NKX2-1 ITGA3 EGFR
10 cytokine-mediated signaling pathway GO:0019221 9.8 TGFB1 MUC1 IL1A CXCL8 CCL5 CCL2
11 cellular response to organic cyclic compound GO:0071407 9.79 TGFB1 CCL5 CCL2
12 positive regulation of epithelial cell proliferation GO:0050679 9.78 TGFB1 EGFR CCL5
13 cellular response to interferon-gamma GO:0071346 9.78 EDN1 CCL5 CCL2 CCL17
14 positive regulation of MAP kinase activity GO:0043406 9.77 TGFB1 EGFR EDN1
15 positive regulation of smooth muscle cell proliferation GO:0048661 9.77 EGFR EDN1 CCL5
16 monocyte chemotaxis GO:0002548 9.73 CCL5 CCL2 CCL17
17 protein kinase B signaling GO:0043491 9.71 TGFB1 CCL5 CCL2
18 chemokine-mediated signaling pathway GO:0070098 9.71 CXCL8 CCL5 CCL2 CCL17
19 lymphocyte chemotaxis GO:0048247 9.7 CCL5 CCL2 CCL17
20 lipopolysaccharide-mediated signaling pathway GO:0031663 9.69 TGFB1 CCL5 CCL2
21 cellular response to fibroblast growth factor stimulus GO:0044344 9.67 CXCL8 CCL5 CCL2
22 negative regulation of G protein-coupled receptor signaling pathway GO:0045744 9.66 CXCL8 CCL5
23 neutrophil activation GO:0042119 9.66 CXCL8 CCL5
24 PERK-mediated unfolded protein response GO:0036499 9.65 CXCL8 CCL2
25 cellular response to tumor necrosis factor GO:0071356 9.65 EDN1 CXCL8 CCL5 CCL2 CCL17
26 cerebral cortex cell migration GO:0021795 9.64 NKX2-1 EGFR
27 positive regulation of production of miRNAs involved in gene silencing by miRNA GO:1903800 9.63 TGFB1 EGFR
28 salivary gland morphogenesis GO:0007435 9.62 TGFB1 EGFR
29 response to salt GO:1902074 9.58 TGFB1 EDN1
30 connective tissue replacement involved in inflammatory response wound healing GO:0002248 9.57 TGFB1 IL1A
31 positive regulation of odontogenesis GO:0042482 9.55 TGFB1 EDN1
32 macrophage chemotaxis GO:0048246 9.54 SFTPD CCL5 CCL2
33 respiratory gaseous exchange GO:0007585 9.46 SFTPD SFTPC SFTPB EDN1
34 cellular response to interleukin-1 GO:0071347 9.35 EDN1 CXCL8 CCL5 CCL2 CCL17
35 neutrophil chemotaxis GO:0030593 9.1 IL1A EDN1 CXCL8 CCL5 CCL2 CCL17

Molecular functions related to Interstitial Lung Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 CCR chemokine receptor binding GO:0048020 9.43 CCL5 CCL2 CCL17
2 chemokine activity GO:0008009 9.26 CXCL8 CCL5 CCL2 CCL17
3 cytokine activity GO:0005125 9.17 TGFB1 IL1A EDN1 CXCL8 CCL5 CCL2
4 CCR4 chemokine receptor binding GO:0031729 9.16 CCL5 CCL17

Sources for Interstitial Lung Disease

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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